母親が神経線維腫症を患っている7歳女児の下顎歯肉過形成を臨床的に認めた正中線神経線維腫

A midline neurofibroma, clinically manifested as a mandibular gingival hyperplasia in a seven-year-old girl whose mother suffers from neurofibromatosis

• Mária Eper
• Péter Nagy
• István Gera

抄録

7歳の少女が、下顎の舌側に対称的な両側性の無痛性、非炎症性のびまん性拡大を認め、センメルヴァイス大学歯周病科に紹介された。家族歴から、母親と姉が典型的な特徴的な皮膚病的徴候を有するレックリングハウゼン病を患っていたことが判明し、彼らはNational NF Registryに登録されていた。口腔外検査で唇の突出が認められた．口腔内では，上下の歯の周囲の頬側歯肉は正常であった．本症例にはNF1に特徴的な真皮徴候も口腔粘膜徴候も認められなかった．両親の同意を得て，局所麻酔下で歯肉肥大の舌側固まりを切除し，切除した組織を組織学的に解析した．組織学的には孤発性神経線維腫と診断された．術後の治癒は問題なかった。若くして腫瘍を全摘出したため、定期的に経過観察を行っている。1年半経過しても再発や腫瘍の再成長の徴候は認められていない。パノラマX線写真では骨形態は正常であり，年齢的には混合歯列と歯萌出は認められた．遺伝的に証明されたRecklinghausen病を持つ家族において、特徴的な皮膚学的徴候を伴わない7歳女児の口腔内における正中線両側のびまん性歯肉過成長を呈した本症例は、文献上非常に稀であり、ユニークな症例である。Orv Hetil.2020; 161(22): 924-930.

A seven-year-old girl was referred to the Department of Periodontology of the Semmelweis University with a symmetric bilateral, painless, non-inflammatory diffuse enlargement on the lingual aspects of her lower jaw. The family history revealed that her mother and elder sister had Recklinghausen's disease with typical characteristic dermatological signs and they are registered by the National NF Registry. Extraoral examination revealed an evident protrusion of the lips. Intraorally, the buccal gingiva around the upper and lower teeth appeared normal. The little patient had neither dermal nor oral mucous membrane signs characteristic of NF1. With the consent of her parents, the lingual firm mass of gingival enlargement was excised under local anesthesia and the removed tissue was histologically analyzed. The histology approved the clinical diagnosis as solitary neurofibroma. The postoperative healing was uneventful. Because of her very young age and the subtotal excision of the tumor, the patient has been regularly monitored. After one and a half years, recurrence or other sign of tumor regrowth have not been observed. The panoramic radiograph showed normal bone morphology and an age-related dental status with mixed dentition and undisturbed tooth eruption. The present case with a midline bilateral diffuse gingival overgrowth in the mouth of a 7-year-old girl without any characteristic dermatological signs in a family with genetically proven Recklinghausen's disease is very rare and unique in the literature. Orv Hetil. 2020; 161(22): 924-930.